Pagnesi Matteo, Riccardi Mauro, Savonitto Giulio, Ameri Pietro, Monti Simonetta, Driussi Mauro, Gentile Piero, Specchia Claudia, Oriecuia Chiara, Adamo Marianna, Toma Matteo, Giannoni Alberto, Di Poi Emma, D'Angelo Luciana, Colombo Valentina, Vecchiato Veronica, Airo Edoardo, Imazio Massimo, Garascia Andrea, Sinagra Gianfranco, Giudice Francesco Lo, Metra Marco, Stolfo Davide, Lombardi Carlo Mario
Institute of Cardiology, Department of Medical and Surgical Specialties, Radiological Sciences and Public Health, University of Brescia, Spedali Civili di Brescia, Brescia, Italy.
Institute of Cardiology, Department of Medical and Surgical Specialties, Radiological Sciences and Public Health, University of Brescia, Spedali Civili di Brescia, Brescia, Italy.
Int J Cardiol. 2025 Jul 1;430:133180. doi: 10.1016/j.ijcard.2025.133180. Epub 2025 Mar 20.
Despite the higher incidence of pulmonary arterial hypertension (PAH) in women, sex differences in terms of clinical presentation and prognosis are still uncertain. We assess the impact of sex in patients with PAH.
Sex differences were evaluated in the multicenter FOCUS-PAH registry, that included patients diagnosed with PAH at 7 tertiary care centers between 2001 and 2022. Patients' characteristics and outcomes were compared in men vs. women, and factors independently associated with long-term outcomes were assessed. The primary endpoint was overall survival.
Among 410 included patients, 273 (66.6 %) were women and 137 (33.4 %) were men. Despite similar age, men had more cardio-renal-metabolic comorbidities and history of HIV infection, whereas women had more connective tissue disorders and scleroderma, and worse functional capacity. Dual therapy was more commonly prescribed in women (39.9 % vs. 27.0 %). At multivariable Cox regression analysis, female sex was independently associated with a lower risk of all-cause mortality (adjusted hazard ratio 0.60, 95 % confidence interval 0.38-0.95, p = 0.031). Age, European Society of Cardiology (ESC) / European Respiratory Society (ERS) risk score, glomerular filtration rate and scleroderma were independently associated with long-term mortality in women, whereas only age and ESC/ERS risk score independently predicted this endpoint in men.
In this real-world registry, women represented 2 out of 3 patients with PAH and female sex was independently associated with a lower risk of mortality. There were differences in the variables associated with long-term mortality between sexes, suggesting that individualized approach to risk stratification should also include sex.
尽管肺动脉高压（PAH）在女性中的发病率较高，但临床表现和预后方面的性别差异仍不明确。我们评估性别对PAH患者的影响。
在多中心FOCUS-PAH注册研究中评估性别差异，该研究纳入了2001年至2022年间在7个三级医疗中心被诊断为PAH的患者。比较男性和女性患者的特征及结局，并评估与长期结局独立相关的因素。主要终点为总生存期。
在纳入的410例患者中，273例（66.6%）为女性，137例（33.4%）为男性。尽管年龄相似，但男性有更多的心肾代谢合并症和HIV感染史，而女性有更多的结缔组织病和硬皮病，且功能能力较差。女性更常接受双联治疗（39.9%对27.0%）。在多变量Cox回归分析中，女性性别与全因死亡风险较低独立相关（调整后风险比0.60，95%置信区间0.38 - 0.95，p = 0.031）。年龄、欧洲心脏病学会（ESC）/欧洲呼吸学会（ERS）风险评分、肾小球滤过率和硬皮病与女性的长期死亡率独立相关，而在男性中只有年龄和ESC/ERS风险评分能独立预测这一终点。
在这个真实世界的注册研究中，PAH患者中三分之二为女性，女性性别与较低的死亡风险独立相关。性别之间与长期死亡率相关的变量存在差异，这表明风险分层的个体化方法也应包括性别。
